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Double seronegative myasthenia gravis with anti-LRP 4 antibodies.

Abstract
About 10% of patients with generalized myasthenia gravis do not have detectable antibodies to acetylcholine receptor or muscle specific kinase (double seronegative myasthenia). The presence of anti-low density lipoprotein receptor-related protein 4 antibodies (LRP4 Abs) has recently been reported in variable proportion of double seronegative cases. We report the presenting characteristics of two double seronegative myasthenic patients from Greece with anti-LRP4 antibodies shortly after disease onset. The first patient, a 52-year-old male, presented with a one month history of isolated neck extensor weakness; the second patient is a 52-year-old female with three months history of ocular-bulbar-cervical myasthenic weakness. Both patients presented with mild severity and responded promptly and adequately to pyridostigmine. In the female patient thymic residual tissue was detected on CT of the mediastinum. She underwent thymectomy, and histological examination revealed follicular hyperplasia. This is the first clinical report of the presenting features of newly diagnosed myasthenia with anti-LRP4 antibodies. The clinical and therapeutic implications of the anti-LRP4 antibody positivity remain to be clarified.
AuthorsVasiliki Zouvelou, Paraskevi Zisimopoulou, Michael Rentzos, Nikos Karandreas, Panagiota Evangelakou, Eleftherios Stamboulis, Socrates J Tzartos
JournalNeuromuscular disorders : NMD (Neuromuscul Disord) Vol. 23 Issue 7 Pg. 568-70 (Jul 2013) ISSN: 1873-2364 [Electronic] England
PMID23768983 (Publication Type: Case Reports, Journal Article)
CopyrightCopyright © 2013 Elsevier B.V. All rights reserved.
Chemical References
  • Autoantibodies
  • LDL-Receptor Related Proteins
  • LRP4 protein, human
  • Receptor Protein-Tyrosine Kinases
  • Pyridostigmine Bromide
Topics
  • Autoantibodies (blood)
  • Female
  • Humans
  • LDL-Receptor Related Proteins (blood, immunology)
  • Male
  • Middle Aged
  • Myasthenia Gravis (diagnosis, drug therapy, immunology)
  • Pyridostigmine Bromide (therapeutic use)
  • Receptor Protein-Tyrosine Kinases (immunology)
  • Thymectomy (methods)
  • Treatment Outcome

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